Clinical characteristics of Guillain-Barré syndrome in a tropical country: a Brazilian experience.

OBJECTIVE: To analyze the clinical variants, outcomes, and prognosis of Guillain-Barré syndrome (GBS) in a Brazilian population. MATERIALS AND METHODS: Clinical and laboratory data of 149 cases of GBS diagnosed from 1994 to 2007 were analyzed. RESULTS: Acute inflammatory demyelinating polyneuropathy (AIDP) was the most frequent variant (81.8%) of GBS, followed by acute motor axonal neuropathy (AMAN) (14.7%) and acute motor and sensory axonal neuropathy (AMSAN) (3.3%). The incidence of GBS was 0.3/100,000 for the state of Rio Grande do Norte and cases occurred at a younger age. GBS was preceded by infections, with the axonal variant associated with episodes of diarrheas (P = 0.025). Proximal weakness was more frequent in AIDP, and distal weakness predominant in the axonal variant. Compared to 42.4% of cases with AIDP (P < 0.0001), 84.6% of cases with the axonal variant had nadir in <10 days. Individuals with the axonal variant took longer to recover deambulation (P < 0.0001). The mortality of GBS was 5.3%. CONCLUSION: A predominance of the AIDP variant was seen, and the incidence of the disease decreased with age. As expected, the distribution of weakness correlated with the clinical variants, and individuals with the axonal variant had a poorer prognosis.

Respiratory muscle strength and quality of life in myotonic dystrophy patients.

INTRODUCTION: Studies on quality of life in myotonic dystrophy (MD) are scarce and the relationship between respiratory muscle strength and health-related quality of life (HRQoL) has yet to be determined. The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients. METHODS: Twenty-three patients (13 men, aged 40 ± 16 years) with MD were evaluated for pulmonary function, maximal inspiratory and expiratory pressure (MIP and MEP, respectively), sniff nasal inspiratory pressure (SNIP) and HRQoL using the Short Form (SF-36) quality of life questionnaire. RESULTS: Respiratory muscle strength values were 71 ± 20 cmH2O (64 % predicted), 76 ± 32 cmH2O (70 % predicted), and 79 ± 28 cmH2O (80 % predicted) for MEP, MIP, and SNIP respectively. Significant differences were found in the SF-36 domains of physical functioning 58.7 ± 31.4 vs. 84.5 ± 23 (p < 0.01, 95 % CI = 1.6-39.9) and physical problems 43.4 ± 35.2 vs. 81.2 ± 34 (p < 0.001, 95 % CI = 19.4-6.1) when compared with the reference values. According to single linear regression analysis, MIP explains 29 % of the variance in physical functioning, 18 % of physical problems and 20 % of vitality. CONCLUSIONS: Individuals with MD have reduced expiratory muscle strength. HRQoL may be more impaired in some physical domains, which might be influenced by variations in inspiratory muscle strength.

Anti-ganglioside antibodies and clinical outcome of patients with Guillain-Barré Syndrome in northeast Brazil.

OBJECTIVES: The goal of this study was to investigate the frequency of GM1 antibodies and to assess whether exposure to Campylobacter jejuni was associated with a distinct clinical variant of Guillain-Barré Syndrome (GBS) or disease outcome in Rio Grande do Norte, Brazil. MATERIAL AND METHODS: Forty-one patients with a presumed diagnosis of GBS were enrolled and prospectively studied between June 1994 and November 1999. RESULTS: Anti-GM1 was present in 51.2% (n = 21) of patients. The presence of anti-GM1 was significantly associated with acute axonal motor neuropathy when compared to acute inflammatory demyelinating polyneuropathy (P = 0.01). Patients with anti-GM1 antibodies presented distal muscle involvement and fewer sensory deficits. Age, time to nadir and ventilatory assistance were not associated with anti-GM1 antibodies. Eight out of 21 patients (32%) presented with anti-C. jejuni antibodies. Clinical features were similar for patients with GBS with positive and negative C. jejuni antibodies. Anti-GM1 antibodies were associated with C. jejuni infection (P = 0.0005). Presence of anti-GM1 and C. jejuni antibodies did not indicate a worse prognosis. CONCLUSION: Patients with GBS and anti-GM1 antibodies had more distal muscle weakness, fewer sensory deficits, more axonal degeneration and C. jejuni infection, but these findings were not associated with a worse prognosis.

[Fluctuations in Guillain-Barré syndrome related with intravenous human immunoglobulin (triphasic course): case report]. / Síndrome de Guillain-Barré com flutuações relacionadas ao tratamento com imunoglobulina humana endovenosa. Curso trifásico. Relato de caso.

The authors report the case of a patient with severe Guillain-Barré syndrome (tetraplegic and on mechanical ventilation), that was treated with intravenous immunoglobulin (IVIg), 2 g/Kg. At first, there was clinical improvement, followed by clinical deterioration two weeks later. On the second course of IVIg there was, again, clinical improvement and then deterioration, 65 days after treatment. Finally, on the third course of treatment definitive recovery was achieved and no more relapses happened so far (three years after the treatment). The authors review the literature about fluctuations related to treatment with IVIg. Conclusions are that these patients should be closely observed during the first weeks after IVIg treatment, and that further studies are still necessary to elaborate alternative protocols on the prevention of these cases.

[Lobar cerebral hemorrhage. Clinical aspects, etiology and course. Review of 58 cases]. / Hemorragia cerebral lobar clínica, etiologia, evolução. Revisão de 58 casos.

Fifty eight patients with spontaneous lobar hemorrhage of a 264 patients series with intracerebral hemorrhage seen during a six years period are reviewed. Twenty nine had arterial hypertension, 9 had other etiologies, and in the remaining 20 cases no apparent etiology was detected. Headache was a prominent symptom and occurred in 56%. Seizures occurred in 12%. Coma was infrequent. Clinical findings on admission depended on the location of the hematoma. Lobar hemorrhage occurred most commonly in the parietal region. Mortality rate was 8.6%. Size of hematoma (larger than 4 cm) on CT and altered consciousness on admission correlated with poor outcome.

Hemorragia cerebral lobar clínica, etiologia, evolução. Revisão de 58 casos / Lobar cerebral hemorrhage. Clinical aspects, etiology and course. Review of 58 cases

Fifty eight patients with spontaneous lobar hemorrhage of a 264 patients series with intracerebral hemorrhage seen during a six years period are reviewed. Twenty nine had arterial hypertension, 9 had other etiologies, and in the remaining 20 cases no apparent etiology was detected. Headache was a prominent symptom and occurred in 56%. Seizures occurred in 12%. Coma was infrequent. Clinical findings on admission depended on the location of the hematoma. Lobar hemorrhage occurred most commonly in the parietal region. Mortality rate was 8.6%. Size of hematoma (larger than 4 cm) on CT and altered consciousness on admission correlated with poor outcome.